关键词: alk-1 imt pleural pseudotumor pulmonary neoplasm

来  源:   DOI:10.7759/cureus.63892   PDF(Pubmed)

Abstract:
The uncommon and mysterious pulmonary inflammatory myofibroblastic tumor (PIMT) primarily affects children and young people. PIMT is characterized by the proliferation of myofibroblastic spindle cells mixed with inflammatory cells. It can resemble both benign and malignant disorders, both radiographically and clinically. PIMT typically manifests as a solitary lung tumor. The genesis of the tumor is linked to genetic anomalies, including those related to the ALK gene (anaplastic lymphoma kinase); nonetheless, some cases are not ALK-positive, indicating genetic variability. Clinically, patients may have non-specific symptoms such as cough, chest pain, or hemoptysis, or they may not exhibit any symptoms at all. In these cases, imaging tests may unintentionally reveal unrelated conditions. From a histopathological perspective, PIMT is characterized by a heterogeneous cellular makeup, encompassing lymphocytes, myofibroblasts, plasma cells, and histiocytes, which generally exhibit a fascicular or storiform pattern. The diagnosis is verified using immunohistochemical labeling, molecular research, and histological examination. The cornerstone of treatment is still surgical resection, which has a good prognosis and a low recurrence rate. On the other hand, specific treatments, such as ALK inhibitors, have shown promise for incurable or recurring instances. Even though PIMT usually has a benign history, it is important to comprehend its biological behavior and molecular foundations for precise diagnosis and efficient management. This underscores the need for additional study into the pathophysiology and potential treatments of PIMT. This report presents a case of a 53-year-old female who presented with complaints of breathlessness and chest pain and was diagnosed with the condition accidentally.
摘要:
罕见且神秘的肺部炎性肌纤维母细胞瘤(PIMT)主要影响儿童和年轻人。PIMT的特征在于与炎性细胞混合的成肌纤维梭形细胞的增殖。它可以类似于良性和恶性疾病,影像学和临床。PIMT通常表现为孤立性肺肿瘤。肿瘤的发生与遗传异常有关,包括与ALK基因(间变性淋巴瘤激酶)相关的基因;尽管如此,有些病例不是ALK阳性,表明遗传变异性。临床上,患者可能有咳嗽等非特异性症状,胸痛,或者咯血,或者他们可能根本没有任何症状。在这些情况下,影像学检查可能无意中发现无关的情况.从组织病理学的角度来看,PIMT的特征是异质细胞组成,包括淋巴细胞,肌成纤维细胞,浆细胞,和组织细胞,通常表现出束状或柱状图案。通过免疫组织化学标记证实诊断,分子研究,和组织学检查。治疗的基石仍然是手术切除,预后良好,复发率低。另一方面,具体治疗,如ALK抑制剂,对无法治愈或反复出现的情况显示出希望。尽管PIMT通常有良性病史,了解其生物学行为和分子基础对于精确诊断和有效管理至关重要。这强调需要对PIMT的病理生理学和潜在治疗进行进一步研究。本报告介绍了一例53岁的女性,她抱怨呼吸困难和胸痛,并被意外诊断出患有这种疾病。
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